Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Like Disease and/or Atopic Dermatitis

Recruiting now · Phase 1

Conditions studied: Hyper IgE Syndrome From STAT3 Mutation, Job s Syndrome, HIES, Lupus, Atopic Dermatitis

In brief

Background: Autosomal dominant hyper-IgE syndrome (HIES), also called Job syndrome, is a genetic disorder that affects the immune system. It can cause skin and lung infections and problems with blood vessels, connective tissues, and bones. People with HIES often have lupus-like disease or atopic dermatitis (skin rash). Researchers want to know if a drug approved to treat other immune system diseases (baricitinib) can help people with HIES. Objective: To test baricitinib in people with HIES with lupus-like disease or skin rash. Eligibility: People aged 12 years and older with HIES with lupus-like disease or skin rash. Design: Participants will have 5 clinic visits, 4 remote visits, and 2 phone visits in 9 months. Participants will be screened. They will have a physical exam with blood and urine tests. They will have tests of the speed and pressure of blood flow through their body: Blood pressure cuffs will be placed on each arm and leg; electrodes will be placed on the wrists and a microphone on the chest. The study has a 3-month lead-in period. Participants will not take the study drug during this time. They will continue with their usual medical care. They will have 2 phone calls with the study team. Baricitinib is a tablet taken by mouth. Participants will take 1 or 2 tablets by mouth every day for 6 months. They will start with a low dose and may increase to a higher dose. Blood and urine tests will be repeated during each study visit. Other tests may also be repeated during some visits. A skin sample may also be taken....

Key facts

Study ID

NCT07262983

Run by

National Institute of Allergy and Infectious Diseases (NIAID)

People needed

20

Starts

2026-08-13

Expected to finish

2030-10-01

Last updated by the study team

2026-08-10

Who can join

Age: 12 and older, up to 120. Sex: any. Healthy volunteers: not accepted.

You may not qualify if…

• An individual who meets any of the following criteria will be excluded from participation in this study:
• Known history of hypersensitivity to baricitinib or other JAK inhibitors.
• Current or recent use of any investigational drug/intervention (within 6 months or 5 half-lives, whichever is longer, prior to Day 0) except for COVID-19 vaccines or therapies that have been granted an FDA emergency authorization.
• Scheduled to participate in another clinical study involving an investigational drug during the course of this study.
• Use of systemic immunosuppressive or immune-modulating agents within 90 days prior to Day 0, except systemic steroids <=10 mg of prednisone equivalent per day.
• Current or prior treatment with rituximab in the 6 months prior to Day 0.
• Current treatment with methotrexate, mycophenolate mofetil, other less common immunomodulatory drugs such as those falling into the class of disease-modifying antirheumatic drugs (DMARDs), belimumab, and other immunosuppressive biologics not otherwise specified herein. Participants previously on methotrexate, mycophenolate mofetil, azathioprine, tacrolimus, cyclosporine, or belimumab, other immunosuppressive biologics, or DMARDs should have been withdrawn from the drug for at least 90 days prior to Day 0.
• Treatment with cyclophosphamide and pulse methylprednisolone within 6 months prior to Day 0.
• Hypercholesterolemia: Values after 8- to 12-hour fasting blood specimen: total cholesterol >250 mg/dL or LDL >180 mg/dL or hypertriglyceridemia (triglyceride >300 mg/dL) within 90 days prior to Day 0.
• History of alcohol or drug abuse within 6 months prior to Day 0.
• Presence of 1 or more of the following clinically significant laboratory abnormalities:
• Serum ALT >=3 times ULN.
• Serum total bilirubin >=2 times ULN.
• ANC <=750 cells/microL.
• Hemoglobin <=9.0 g/dL.
• Platelet count <=100,000/microL.
• Serum creatinine >=2 times ULN.
• Planned or anticipated major surgical procedure during the study.
• Plans to receive any live vaccines within 1 month of the anticipated first dose of baricitinib.
• Known or suspected immune-dysregulatory disorders besides Job s syndrome, lupus-like disease, and/or AD.
• Active invasive opportunistic infections (eg, non-TB mycobacterial infections, histoplasmosis, listeriosis, coccidioidomycosis, pneumocystis pneumonia, aspergillosis) despite infection resolution or otherwise recurrent infections of abnormal frequency or prolonged infections suggesting an immune-compromised status as judged by the investigator.
• Known active TB. Participants with treated LTB will be eligible to participate. Participants with untreated LTB will not be excluded but will be evaluated by an infectious disease consultant and may become eligible for trial based on infectious disease consultant recommendations.
• Infection with HIV.
• Untreated infection with hepatitis B or C.
• Unwillingness to receive prophylactic entecavir (or similar), only for individuals with evidence of clearance of hepatitis B with positive hepatitis B core and surface antibody and negative hepatitis B surface antigen and PCR.

Where it is running

• National Institutes of Health Clinical Center — Bethesda, Maryland, United States (enrolling)

Full record on ClinicalTrials.gov

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